Pediatric splanchnic arterial occlusive disease is uncommon and a rare cause of clinically relevant intestinal ischemia. This study was undertaken to better define the clinical manifestations and appropriate treatment of celiac artery (CA) and superior mesenteric artery (SMA) occlusive disease in children.Clinical courses of 30 consecutive children undergoing operations for splanchnic arterial occlusive disease at the University of Michigan from 1992 to 2017 were retrospectively analyzed.Vascular reconstructions were performed for splanchnic arterial disease in 18 boys and 12 girls, 1.5 to 16 years of age (mean, 7.5 ± 4.1 years). Isolated splanchnic arterial disease was uncommon (2 children), being more often associated with abdominal aortic coarctations (19 children) or ostial renal artery stenoses (25 children). Primary splanchnic arterial reconstructions (30) included aortic reimplantation of SMAs (15) or celiacomesenteric arteries (2), aortoceliac and aortomesenteric bypasses (7), reimplantation of the CA as a patch over the stenotic SMA orifice (3), and patch angioplasty of the CA (2) or SMA (1). There was no perioperative mortality. Two groups (I and II) were identified for study. Group I children (14) experienced symptomatic intestinal ischemia, manifested by various combinations of chronic postprandial abdominal discomfort (14), ischemia-related intestinal bleeding (2), or failure to thrive (4). Four children in group I became symptomatic after known CA and SMA occlusive disease was left untreated at the time they underwent earlier interventions for renovascular hypertension. Seven secondary redo interventions were undertaken for recurrent symptoms in six group I children. Only one major periprocedural complication occurred: segmental colon infarction. The assisted patency rate of reconstructed arteries in group I children was 93%, and intestinal ischemic symptoms resolved in every child. Group I follow-up from the most recent splanchnic arterial reconstruction averaged 4.3 years. Group II children (16) without manifestations of intestinal ischemia underwent prophylactic splanchnic arterial reconstructions in concert with combined aortic and renal artery procedures (11), isolated abdominal aortic reconstructions (3), or renal artery reconstructions alone (2). Group II children experienced no major perioperative morbidity and remained asymptomatic postoperatively, and none required secondary splanchnic artery interventions. Group II follow-up averaged 7.4 years.Pediatric splanchnic artery occlusive lesions are often associated with developmental aortic and renal artery occlusive disease. Carefully conducted therapeutic and prophylactic reconstructive procedures are appropriate in children having splanchnic arterial occlusive disease.